Successful treatment of fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions

Abstract

BackgroundEcthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18 expression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal infections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection.Case PresentationWe report at our knowledge the first case of a child affected by LAD-1, who experienced during her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted antibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes pockets transfusions in association with G-CSF had the capacity to cure this lesion.ConclusionWe propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.

Genetic Susceptibility to Fungal Infections in Humans

“…IMIs are occasionally encountered in autosomal recessive leukocyte adhesion deficiency type 1 (LAD-1), caused by mutations in ITGB2 that encodes the β2-integrin common chain, leading to diminished granulocyte adhesion and migration [30]. Similarly, IMIs develop in LAD-1/variant syndrome, a distinct entity comprising a moderate LAD-1–like phenotype with a Glanzmann-like bleeding tendency [31].…”

Disseminated Fusarium infection in autologous stem cell transplant recipient

“…Although its efficacy is not well established, some authors suggest a beneficial effect, based on uncontrolled case reports and case series. 17,18 In conclusion, our report illustrates a rare case of a lethal, disseminated Fusarium infection in an autologous BMT recipient during pre-engraftment phase. This unexpected manifestation reinforces the need for careful clinical evaluation of BMT candidates, as well as exhaustive vigilance of environmental cleansing procedures.…”

Ecthyma Gangrenosum in a 67-Year-Old Man with Chronic Lymphocytic Leukemia

“…Ecthyma gangrenosum is a skin disease most commonly associated with Pseudomonas aeruginosa septicemia, although a wide variety of Gram-negative and fungal causative organisms have been reported, including Escherichia coli, Vibrio vulnificus, Neisseria meningitidis, Aspergillus, and Fusarium fungi (1,2). Pseudomonas septicemia reflects a state of immunocompromise and most commonly presents in patients with neutropenia, burns, prolonged courses of antibiotics, chemotherapy, complement deficiencies, or hematologic malignancies, as in our patient (3,4).…”